Mature T-Cell and NK-Cell Neoplasm (2013)

Intro

 

-       Guideline refers to lymphomas by WHO 2008 classification, watch out for differences

-       Mature or peripheral T-cell neoplasms are biologically and clinically varied

-       Result from clonal proliferation of post-thymic lymphocytes

-       Good prognosis = Anaplastic Lymphoma Kinase (ALK) positive anaplastic large cell (ALCL)

-       Most nodal and extra-nodal T-cell lymphomas however have 30% 5-year survival

-       Rare and lack phase 3 trial data due to low numbers.

 

WHO 2016 Classification

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Epidemiology

 

10% of lymphoid malignancies

Incidence 1.7 / 100,000 / year

NK-Cell lymphomas more common in Asia and associated with EBV

Adult T-Cell leukaemia/lymphoma (ATL) associated with HTLV-1

 

Presentation & Diagnosis

 

Often present late with advanced stage, poor performance status and extra-nodal disease

Para-neoplastic features seen – eosinophilia, HLH, autoimmune processes

FBC, Biochem, LDH, B2M, CXR, CT, BM Biopsy

Role of PET-CT still under investigation

 

Mature T-Cell Leukaemias

 

T-Prolymphocytic Leukaemia (T-PLL)

 

-       Association with ataxia telangiectasia

-       Aggressive disease present with WBC >100, splenomegaly and lymphadenopathy

-       Mature T cell markers – CD2+, CD3 weak+, CD5+, CD7+

-       OS 7 months

-       1st line therapy: Alemtuzumab. Transplant in first remission.

 

T-Large Granular Lymphocytic Leukaemia (T-LGL)

 

-       Associated with AI disorders, e.g. rheumatoid arthritis

-       Indolent, median survival >10 years

-       Most patients develop severe neutropenia

-       Markers – CD3+, TCRαβ+, CD8+, CD16+, CD57+, CD94+, KIR+, CD56-

-       Treat if complications from cytopenias

-       1st line: Ciclosporin or low dose methotrexate + GCSF/Epo

 

Aggressive NK-cell leukaemia

 

-       More common in Asia and almost always associated with EBV

-       Acute presentation with fever, jaundice, LNadenopathy, organomegaly

-       Patients are sick, may have DIC / HLH / MOF

-       Markers – CD3episilon+, CD56+, CD16+, CD2-, sCD3-, CD57-

-       OS 2 months

-       1st line: Clinical trial, ALL-type therapy

 

Adult T-Cell Lymphoma (ATL)

 

-       Caused by HTLV-1, which is epidemic in Japan, Africa, South America

-       Check for strongyloides at diagnosis

-       4 subtypes – leukaemic, lymphoma, chronic & smouldering

-       Markers – CD4+, CD25+, CD7-

-       1st line therapy depends on type

o   Smouldering & Chronic – anti-retrovirals +/- monoclonal antibodies

o   Lymphoma – CHOP + antivirals + monoclonal antibodies

o   Leukaemia - CHOP + antivirals + monoclonal antibodies. Then Transplant

 

Nodal PTCL

 

Peripheral T-Cell Lymphoma, NOS (PTCL-NOS)

 

-       Largest group, 50% of T-cell cases

-       Aggressive, usually nodal

-       1st line therapy: Clinical trial, otherwise CHOP but disappointing results. Autograft in 1st CR.

 

Angio-Immunoblastic T Cell Lymphoma (AITL)

 

-       A disease of the elderly

-       Presentation often mimics an infectious process

-       Sometimes spontaneously regresses

-       OS 30% at 5-years

-       1st line therapy: Trial, otherwise CHOP or FC

 

Anaplastic Large Cell Lymphoma (ALCL)

 

-       Anaplastic lymphoma kinase (ALK) is a receptor tyrosine kinase, usually only found in CNS.

-       NPM1-ALK fusion gene defines ALK+ ALCL

-       ALK+ significantly better prognosis than ALK-. Though ALK- still better than PTCL-NOS

-       1st line therapy: Clinical trial or CHOP. Autograft

-       Cutaneous ALCL can have local excision +/- radiotherapy

 

Extra-Nodal PTCL

 

Extranodal NK/T-Cell Lymphoma, nasal type

 

-       Aggressive, EBV associated

-       Presents in nasal structure but nodal or BM involvement can be present

-       Presents as adult male with facial oedema, nasal obstruction and epistaxis

-       1st line therapy: Trial, otherwise CHOP + radiotherapy but this is unsatisfactory

 

Enteropathy-Associated T-Cell Lymphoma (EATL)

 

-       Aggressive large cell tumour of the small bowel

-       Associated with HLA-DQ 2or 8, and coeliac disease

-       Presents as older male with diarrhoea and abdominal pain

-       OS 7 months

-       1st line therapy: No satisfactory treatment. CHOP + Autograft

 

Hepatosplenic T-Cell Lymphoma

 

-       Aggressive disease of young adult males

-       Associated with post solid organ transplant or immunosuppression

-       OS 16 months

-       1st line therapy: No satisfactory recommendations can be made

 

Subcutaneous pannciulitis-like T-Cell Lymphoma (SPTCL)

 

-       Presents as subcutaneous tumour nodules at any age.

-       Not universally aggressive. Consider CHOP / radiotherapy